Primary paediatric cutaneous mucinoses Franco Rongioletti Unit of Dermatology, University of Cagliari, Italy British Journal of Dermatology. DOI: 10.1111/bjd.18004

Franco Rongioletti

Introduction What’s already known? The nosography of primary cutaneous mucinoses occurring in childhood is confusing because of the very limited number of cases and overlaps in their clinicopathological features in the absence of homogeneous case series. Their diagnosis and management is also difficult as they are diseases of adult age, rarely seen in children

Objective The aim of this review is to help dermatologists and paediatricians identify patients with cutaneous mucinoses in paediatric age and enable institution of appropriate management. A classification of primary paediatric cutaneous mucinoses is proposed.

Methods We review the cutaneous mucinoses typically seen in paediatric age, their diagnostic evaluations, prognosis and management. A classification of primary paediatric cutaneous mucinoses is proposed

Results The classification

Cutaneous mucinosis of infancy (CMI) Self healing juvenile cutaneous mucinosis (SHJCM) Scleredema

Follicular mucinosis (FM) Mucinous nevus (MN) (Angio)myxoma

Discussion The paediatric cutaneous mucinoses are divided into 2 groups: primary (idiopathic) cutaneous mucinoses in which the mucin deposit is the main histologic feature resulting in clinically distinctive lesions and secondary mucinoses in which histologic mucin deposition is only an additional finding and epiphenomenon. They can be further divided into degenerative/inflammatory and hamartomatous/neoplastic (Table 1), both dermal and follicular.

Discussion The degenerative/inflammatory paediatric mucinoses include substantially a benign form without systemic symptoms such as CMI that does not tend to resolve and two benign forms which are self-healing such as SHJCM and scleredema, even though they may associate systemic symptoms. Primary follicular mucinosis should be distinguished from follicular mucinosis associated with cutaneous T-cell lymphoma The hamartomatous/neoplastic mucinoses include mostly MN which is a rare form of connective tissue nevus and (angio)myxoma which is a true benign neoplasm .

Conclusions What does this study add? New investigations have yielded significant insights into a classification of paediatric cutaneous mucinoses The current update tries to offer more meaningful guide to diagnosis and treatment of these rare diseases in paediatric age

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